2J25

Partially deglycosylated glucoceramidase

2J25 の概要

• エントリーDOI: 10.2210/pdb2j25/pdb
• 関連するPDBエントリー: 1OGS 1Y7V 2F61
• 分子名称: GLUCOSYLCERAMIDASE, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, alpha-D-mannopyranose-(1-3)-[alpha-D-mannopyranose-(1-6)]beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... (7 entities in total)
• 機能のキーワード: pharmaceutical, gaucher disease, disease mutation, glycosidase, sphingolipid, polymorphism, glycoprotein, membrane, lysosome, hydrolase, glucosidase, glucocerebrosidase, alternative initiation, lipid metabolism, cerezyme hydrolase, sphingolipid metabolism
• 由来する生物種: HOMO SAPIENS (HUMAN)
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 115480.78

構造登録者

Brumshtein, B.,Wormald, M.R.,Silman, I.,Futerman, A.H.,Sussman, J.L. (登録日: 2006-08-16, 公開日: 2006-12-06, 最終更新日: 2024-10-09)

主引用文献

Brumshtein, B.,Wormald, M.R.,Silman, I.,Futerman, A.H.,Sussman, J.L.
Structural Comparison of Differently Glycosylated Forms of Acid-Beta-Glucosidase, the Defective Enzyme in Gaucher Disease
Acta Crystallogr.,Sect.D, 62:1458-, 2006

PubMed Abstract: Gaucher disease is caused by mutations in the gene encoding acid-beta-glucosidase. A recombinant form of this enzyme, Cerezyme, is used to treat Gaucher disease patients by ;enzyme-replacement therapy'. Crystals of Cerezyme after its partial deglycosylation were obtained earlier and the structure was solved to 2.0 A resolution [Dvir et al. (2003), EMBO Rep. 4, 704-709]. The crystal structure of unmodified Cerezyme is now reported, in which a substantial number of sugar residues bound to three asparagines via N-glycosylation could be visualized. The structure of intact fully glycosylated Cerezyme is virtually identical to that of the partially deglycosylated enzyme. However, the three loops at the entrance to the active site, which were previously observed in alternative conformations, display additional variability in their structures. Comparison of the structure of acid-beta-glucosidase with that of xylanase, a bacterial enzyme from a closely related protein family, demonstrates a close correspondence between the active-site residues of the two enzymes.

PubMed: 17139081
DOI: 10.1107/S0907444906038303

実験手法

X-RAY DIFFRACTION (2.9 Å)